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@article{RN77,
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@article{RN116,
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@inbook{RN62,
   author = {Kaler, Stephen G},
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@article{RN126,
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@book{RN110,
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@article{RN201,
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@article{RN220,
   author = {C Ubah, Obinna and M Wallace, Heather},
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@article{RN170,
   author = {Woronowicz, Alicja and Cawley, Niamh X and Chang, Su‐Youne and Koshimizu, Hisatsugu and Phillips, Andrè W and Xiong, Zhi‐Gang and Loh, Y Peng },
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@article{RN85,
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   title = {Cell‐Specific Trafficking Suggests a new role for Renal ATP7B in the Intracellular Copper Storage},
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   pages = {767-779},
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@article{RN24,
   author = {Hasinoff, Brian B and Wu, Xing and Yadav, Arun A and Patel, Daywin and Zhang, Hui and Wang, De-Shen and Chen, Zhe-Sheng and Yalowich, Jack C},
   title = {Cellular mechanisms of the cytotoxicity of the anticancer drug elesclomol and its complex with Cu (II)},
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@article{RN78,
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   title = {Cerebrospinal Fluid-Directed rAAV9-rsATP7A Plus Subcutaneous Copper Histidinate Advance Survival and Outcomes in a Menkes Disease Mouse Model},
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@article{RN162,
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   title = {Characterization of COX17, a yeast gene involved in copper metabolism and assembly of cytochrome oxidase},
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@article{RN61,
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@article{RN53,
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@article{RN91,
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@article{RN81,
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@article{RN52,
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@article{RN108,
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@article{RN17,
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   pages = {185-211},
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@article{RN48,
   author = {Vest, Katherine E and Leary, Scot C and Winge, Dennis R and Cobine, Paul A},
   title = {Copper import into the mitochondrial matrix in Saccharomyces cerevisiae is mediated by Pic2, a mitochondrial carrier family protein},
   journal = {Journal of Biological Chemistry},
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   pages = {23884-23892},
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@article{RN182,
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   title = {Copper is taken up efficiently from albumin and alpha-2-macroglobulin by cultured human cells by more than one mechanism},
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@article{RN181,
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@article{RN183,
   author = {Vargas, EDWARD J and Shoho, ANGELA R and Linder, MARIA C},
   title = {Copper transport in the Nagase analbuminemic rat},
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@article{RN177,
   author = {Kuo, Yien-Ming and Gybina, Anna A and Pyatskowit, Joshua W and Gitschier, Jane and Prohaska, Joseph R},
   title = {Copper transport protein (Ctr1) levels in mice are tissue specific and dependent on copper status},
   journal = {The Journal of nutrition},
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@article{RN187,
   author = {Kuo, Yien-Ming and Gybina, Anna A and Pyatskowit, Joshua W and Gitschier, Jane and Prohaska, Joseph R},
   title = {Copper transport protein (Ctr1) levels in mice are tissue specific and dependent on copper status},
   journal = {The Journal of nutrition},
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@article{RN176,
   author = {Tsai, Cheng-Yu and Liebig, Janika K and Tsigelny, Igor F and Howell, Stephen B},
   title = {The copper transporter 1 (CTR1) is required to maintain the stability of copper transporter 2 (CTR2)},
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@article{RN79,
   author = {Kuo, Yien-Ming and Zhou, Bing and Cosco, Dominique and Gitschier, Jane},
   title = {The copper transporter CTR1 provides an essential function in mammalian embryonic development},
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@article{RN225,
   author = {Kim, BE and Smith, K and Petris, MJ},
   title = {A copper treatable Menkes disease mutation associated with defective trafficking of a functional Menkes copper ATPase},
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@article{RN112,
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@article{RN172,
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@article{RN113,
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@article{RN50,
   author = {Hiser, Laree and Di Valentin, Marilena and Hamer, Alicia G and Hosler, Jonathan P},
   title = {Cox11p Is Required for Stable Formation of the CuBand Magnesium Centers of Cytochrome c Oxidase},
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@article{RN82,
   author = {Nose, Yasuhiro and Kim, Byung-Eun and Thiele, Dennis},
   title = {Ctr1 drives intestinal copper absorption and is essential for growth, iron metabolism, and neonatal cardiac function},
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@article{RN178,
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   title = {Ctr1 is an apical copper transporter in mammalian intestinal epithelial cells in vivo that is controlled at the level of protein stability},
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@article{RN28,
   author = {Hasinoff, Brian B and Yadav, Arun A and Patel, Daywin and Wu, Xing},
   title = {The cytotoxicity of the anticancer drug elesclomol is due to oxidative stress indirectly mediated through its complex with Cu (II)},
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@article{RN120,
   author = {Wyman, Steven and Simpson, Robert J and McKie, Andrew T and Sharp, Paul A},
   title = {Dcytb (Cybrd1) functions as both a ferric and a cupric reductase in vitro},
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@article{RN174,
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@article{RN94,
   author = {Kim, Heejeong and Son, Hwa-Young and Bailey, Sarah M and Lee, Jaekwon},
   title = {Deletion of hepatic Ctr1 reveals its function in copper acquisition and compensatory mechanisms for copper homeostasis},
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@article{RN196,
   author = {Kim, Heejeong and Son, Hwa-Young and Bailey, Sarah M and Lee, Jaekwon},
   title = {Deletion of hepatic Ctr1 reveals its function in copper acquisition and compensatory mechanisms for copper homeostasis},
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@article{RN184,
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@article{RN97,
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@article{RN90,
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@article{RN21,
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@article{RN188,
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@article{RN75,
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@article{RN18,
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@article{RN123,
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@article{RN106,
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@article{RN124,
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@article{RN25,
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@article{RN93,
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@article{RN73,
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@article{RN107,
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@article{RN45,
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@article{RN11,
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@article{RN60,
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@article{RN148,
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@article{RN80,
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@article{RN105,
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@article{RN111,
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@article{RN101,
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@phdthesis{RN23,
   author = {Helsel, Marian Elizabeth},
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   type = {Thesis}
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@article{RN41,
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   title = {Final results of phase III SYMMETRY study: randomized, double-blind trial of elesclomol plus paclitaxel versus paclitaxel alone as treatment for chemotherapy-naive patients with advanced melanoma},
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@article{RN141,
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@article{RN102,
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@article{RN103,
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@article{RN72,
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@article{RN40,
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@article{RN87,
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   year = {1982},
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}

@article{RN218,
   author = {Ramos, Danny and Mar, David and Ishida, Michael and Vargas, Rebecca and Gaite, Michaella and Montgomery, Aaron and Linder, Maria C},
   title = {Mechanism of copper uptake from blood plasma ceruloplasmin by mammalian cells},
   journal = {PLoS One},
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   pages = {e0149516},
   ISSN = {1932-6203},
   year = {2016},
   type = {Journal Article}
}

@article{RN22,
   author = {Kussmaul, Lothar and Hirst, Judy},
   title = {The mechanism of superoxide production by NADH: ubiquinone oxidoreductase (complex I) from bovine heart mitochondria},
   journal = {Proceedings of the National Academy of Sciences },
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   year = {2006},
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}

@article{RN109,
   author = {Kim, Byung-Eun and Nevitt, Tracy and Thiele, Dennis J},
   title = {Mechanisms for copper acquisition, distribution and regulation},
   journal = {Nature chemical biology},
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   number = {3},
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   year = {2008},
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}

@article{RN145,
   author = {Caruano-Yzermans, Amy L and Bartnikas, Thomas B and Gitlin, Jonathan D},
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   volume = {281},
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   year = {2006},
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}

@article{RN219,
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@article{RN180,
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@article{RN71,
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   journal = {Nature genetics},
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@article{RN127,
   author = {Portnoy, M and Schmidt, P and Rogers, R and Culotta, V},
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}

@article{RN125,
   author = {Selvaraj, Anand and Balamurugan, Kuppusamy and Yepiskoposyan, Hasmik and Zhou, Hao and Egli, Dieter and Georgiev, Oleg and Thiele, Dennis J and Schaffner, Walter},
   title = {Metal-responsive transcription factor (MTF-1) handles both extremes, copper load and copper starvation, by activating different genes},
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}

@article{RN147,
   author = {Hamza, Iqbal and Faisst, Anja and Prohaska, Joseph and Chen, Joseph and Gruss, Peter and Gitlin, Jonathan D},
   title = {The metallochaperone Atox1 plays a critical role in perinatal copper homeostasis},
   journal = {Proceedings of the National Academy of Sciences },
   volume = {98},
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   year = {2001},
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}

@article{RN155,
   author = {Davis, Steven R and Cousins, Robert J},
   title = {Metallothionein expression in animals: a physiological perspective on function},
   journal = {The Journal of nutrition},
   volume = {130},
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   year = {2000},
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}

@article{RN153,
   author = {Tapia, Lucia and González-Agüero, Mauricio and Cisternas, Mónica F and Suazo, Miriam and Cambiazo, Verónica and Ricardo, UAUY and Gonzalez, Mauricion},
   title = {Metallothionein is crucial for safe intracellular copper storage and cell survival at normal and supra-physiological exposure levels},
   journal = {Biochemical journal },
   volume = {378},
   number = {2},
   pages = {617-624},
   ISSN = {0264-6021},
   year = {2004},
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}

@article{RN56,
   author = {Gurgel-Giannetti, Juliana and Oliveira, Guilherme and Brasileiro Filho, Geraldo and Martins, Poliana and Vainzof, Mariz and Hirano, Michio},
   title = {Mitochondrial cardioencephalomyopathy due to a novel SCO2 mutation in a Brazilian patient: case report and literature review},
   journal = {JAMA neurology},
   volume = {70},
   number = {2},
   pages = {258-261},
   ISSN = {2168-6149},
   year = {2013},
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}

@inproceedings{RN44,
   author = {Timón-Gómez, Alba and Nývltová, Eva and Abriata, Luciano A and Vila, Alejandro J and Hosler, Jonathan and Barrientos, Antoni},
   title = {Mitochondrial cytochrome c oxidase biogenesis: Recent developments},
   booktitle = {Seminars in cell & developmental biology},
   publisher = {Elsevier},
   volume = {76},
   pages = {163-178},
   ISBN = {1084-9521},
   type = {Conference Proceedings}
}

@article{RN29,
   author = {Toogood, Peter L},
   title = {Mitochondrial drugs},
   journal = {Current opinion in chemical biology },
   volume = {12},
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   year = {2008},
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}

@article{RN20,
   author = {Blackman, Ronald K and Cheung-Ong, Kahlin and Gebbia, Marinella and Proia, David A and He, Suqin and Kepros, Jane and Jonneaux, Aurelie and Marchetti, Philippe and Kluza, Jerome and Rao, Patricia E},
   title = {Mitochondrial electron transport is the cellular target of the oncology drug elesclomol},
   journal = {PloS one},
   volume = {7},
   number = {1},
   pages = {e29798},
   ISSN = {1932-6203},
   year = {2012},
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}

@article{RN47,
   author = {Cobine, Paul A and Pierrel, Fabien and Bestwick, Megan L and Winge, Dennis R},
   title = {Mitochondrial matrix copper complex used in metallation of cytochrome oxidase and superoxide dismutase},
   journal = {Journal of Biological Chemistry},
   volume = {281},
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   pages = {36552-36559},
   ISSN = {0021-9258},
   year = {2006},
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}

@article{RN198,
   author = {Hlynialuk, Christopher J and Ling, Binbing and Baker, Zakery N and Cobine, Paul A and Lisa, D Yu and Boulet, Aren and Wai, Timothy and Hossain, Amzad and El Zawily, Amr M and McFie, Pamela J },
   title = {The mitochondrial metallochaperone SCO1 is required to sustain expression of the high-affinity copper transporter CTR1 and preserve copper homeostasis},
   journal = {Cell reports},
   volume = {10},
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   ISSN = {2211-1247},
   year = {2015},
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}

@article{RN84,
   author = {Baker, Zakery N and Jett, Kimberly and Boulet, Aren and Hossain, Amzad and Cobine, Paul A and Kim, Byung-Eun and El Zawily, Amr M and Lee, Ling and Tibbits, Glen F and Petris, Michael J},
   title = {The mitochondrial metallochaperone SCO1 maintains CTR1 at the plasma membrane to preserve copper homeostasis in the murine heart},
   journal = {Human molecular genetics},
   volume = {26},
   number = {23},
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   ISSN = {0964-6906},
   year = {2017},
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}

@article{RN197,
   author = {Baker, Zakery N and Jett, Kimberly and Boulet, Aren and Hossain, Amzad and Cobine, Paul A and Kim, Byung-Eun and El Zawily, Amr M and Lee, Ling and Tibbits, Glen F and Petris, Michael J},
   title = {The mitochondrial metallochaperone SCO1 maintains CTR1 at the plasma membrane to preserve copper homeostasis in the murine heart},
   journal = {Human molecular genetics },
   volume = {26},
   number = {23},
   pages = {4617-4628},
   ISSN = {0964-6906},
   year = {2017},
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}

@article{RN164,
   author = {Baker, Zakery N and Cobine, Paul A and Leary, Scot C},
   title = {The mitochondrion: a central architect of copper homeostasis},
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   volume = {9},
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   year = {2017},
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}

@article{RN203,
   author = {Roh, Hyun Cheol and Dimitrov, Ivan and Deshmukh, Krupa and Zhao, Guoyan and Warnhoff, Kurt and Cabrera, Daniel and Tsai, Wendy and Kornfeld, Kerry},
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   year = {2014},
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}

@article{RN151,
   author = {Antala, Sagar},
   title = {Molecular insights of the human zinc transporter hZIP4},
   year = {2016},
   type = {Journal Article}
}

@article{RN14,
   author = {Yadav, Arun A and Patel, Daywin and Wu, Xing and Hasinoff, Brian},
   title = {Molecular mechanisms of the biological activity of the anticancer drug elesclomol and its complexes with Cu (II), Ni (II) and Pt (II)},
   journal = {Journal of inorganic biochemistry},
   volume = {126},
   pages = {1-6},
   ISSN = {0162-0134},
   year = {2013},
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}

@article{RN128,
   author = {Tsai, Cheng-Yu and Larson, Christopher A and Safaei, Roohangiz and Howell, Stephen B},
   title = {Molecular modulation of the copper and cisplatin transport function of CTR1 and its interaction with IRS-4},
   journal = {Biochemical pharmacology},
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   pages = {379-387},
   ISSN = {0006-2952},
   year = {2014},
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}

@article{RN211,
   author = {Harris, Gareth and Mills, Holly and Wragg, Rachel and Hapiak, Vera and Castelletto, Michelle and Korchnak, Amanda and Komuniecki, Richard},
   title = {The monoaminergic modulation of sensory-mediated aversive responses in Caenorhabditis elegans requires glutamatergic/peptidergic cotransmission},
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}

@article{RN67,
   author = {Lenartowicz, Małgorzata and Krzeptowski, Wojciech and Lipiński, Paweł and Grzmil, Paweł and Starzyński, Rafał and Pierzchała, Olga and Møller, Lisbeth Birk},
   title = {Mottled mice and non-mammalian models of Menkes disease},
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}

@article{RN68,
   author = {Cecchi, Chiara and Biasotto, Michel and Tosi, Mario and Avner, Philip},
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}

@book{RN213,
   author = {Wahlsten, Douglas},
   title = {Mouse behavioral testing: how to use mice in behavioral neuroscience},
   publisher = {Academic Press},
   ISBN = {0123756758},
   year = {2010},
   type = {Book}
}

@article{RN7,
   author = {Hüttemann, Maik and Pecina, Petr and Rainbolt, Matthew and Sanderson, Thomas H and Kagan, Valerian E and Samavati, Lobelia and Doan, Jeffrey W and Lee, Icksoo %J Mitochondrion},
   title = {The multiple functions of cytochrome c and their regulation in life and death decisions of the mammalian cell: From respiration to apoptosis},
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}

@article{RN58,
   author = {Baertling, Fabian and AM van den Brand, Mariel and Hertecant, Jozef L and Al‐Shamsi, Aisha and P van den Heuvel, Lambert and Distelmaier, Felix and Mayatepek, Ertan and Smeitink, Jan A and Nijtmans, Leo GJ and Rodenburg, Richard JT},
   title = {Mutations in COA 6 cause Cytochrome c Oxidase Deficiency and Neonatal Hypertrophic Cardiomyopathy},
   journal = {Human mutation},
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}

@article{RN55,
   author = {Valnot, Isabelle and Osmond, Sandrine and Gigarel, Nadine and Mehaye, Blandine and Amiel, Jeanne and Cormier-Daire, Valérie and Munnich, Arnold and Bonnefont, Jean-Paul and Rustin, Pierre and Rötig, Agnès},
   title = {Mutations of the SCO1 gene in mitochondrial cytochrome c oxidase deficiency with neonatal-onset hepatic failure and encephalopathy},
   journal = {The American Journal of Human Genetics},
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}

@article{RN227,
   author = {Mitani, Shohei},
   title = {Nematode, an experimental animal in the national BioResource project},
   journal = {Experimental animals},
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   pages = {351-356},
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   year = {2009},
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}

@article{RN69,
   author = {Kaler, Stephen G and Holmes, Courtney S and Goldstein, David S and Tang, Jingrong and Godwin, Sarah C and Donsante, Anthony and Liew, Clarissa J and Sato, Susumu and Patronas, Nicholas},
   title = {Neonatal diagnosis and treatment of Menkes disease},
   journal = {New England Journal of Medicine},
   volume = {358},
   number = {6},
   pages = {605-614},
   ISSN = {0028-4793},
   year = {2008},
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}

@article{RN131,
   author = {Banci, Lucia and Bertini, Ivano and Ciofi‐Baffoni, Simone and Chasapis, Christos T and Hadjiliadis, Nick and Rosato, Antonio},
   title = {An NMR study of the interaction between the human copper (I) chaperone and the second and fifth metal‐binding domains of the Menkes protein},
   journal = {The FEBS journal},
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   pages = {865-871},
   ISSN = {1742-464X},
   year = {2005},
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}

@article{RN205,
   author = {Freedman, Jonathan H and Slice, Lee W and Dixon, Dennis and Fire, Andrew and Rubin, Charles S},
   title = {The novel metallothionein genes of Caenorhabditis elegans. Structural organization and inducible, cell-specific expression},
   journal = {Journal of Biological Chemistry},
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@article{RN83,
   author = {Turski, Michelle L and Brady, Donita C and Kim, Hyung J and Kim, Byung-Eun and Nose, Yasuhiro and Counter, Christopher M and Winge, Dennis R and Thiele, Dennis J},
   title = {A novel role for copper in Ras/mitogen-activated protein kinase signaling},
   journal = {Molecular and cellular biology},
   volume = {32},
   number = {7},
   pages = {1284-1295},
   ISSN = {0270-7306},
   year = {2012},
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}

@article{RN150,
   author = {Itoh, Shinichi and Kim, Ha Won and Nakagawa, Osamu and Ozumi, Kiyoshi and Lessner, Susan M and Aoki, Hiroki and Akram, Kamran and McKinney, Ronald D and Ushio-Fukai, Masuko and Fukai, Tohru},
   title = {Novel role of antioxidant-1 (Atox1) as a copper-dependent transcription factor involved in cell proliferation},
   journal = {Journal of Biological Chemistry},
   volume = {283},
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   pages = {9157-9167},
   ISSN = {0021-9258},
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}

@article{RN204,
   author = {Warnhoff, Kurt and Roh, Hyun C and Kocsisova, Zuzana and Tan, Chieh-Hsiang and Morrison, Andrew and Croswell, Damari and Schneider, Daniel L and Kornfeld, Kerry},
   title = {The nuclear receptor HIZR-1 uses zinc as a ligand to mediate homeostasis in response to high zinc},
   journal = {PLoS biology},
   volume = {15},
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}

@book{RN95,
   author = {Klurfeld, David M},
   title = {Nutrition and immunology},
   publisher = {Springer Science & Business Media},
   ISBN = {146152900X},
   year = {2013},
   type = {Book}
}

@article{RN122,
   author = {Maryon, Edward B and Molloy, Shannon A and Kaplan, Jack H},
   title = {O-linked glycosylation at threonine 27 protects the copper transporter hCTR1 from proteolytic cleavage in mammalian cells},
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   year = {2007},
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}

@article{RN16,
   author = {Nagai, Masazumi and Vo, Nha H and Ogawa, Luisa Shin and Chimmanamada, Dinesh and Inoue, Takayo and Chu, John and Beaudette-Zlatanova, Britte C and Lu, Rongzhen and Blackman, Ronald K and Barsoum, James},
   title = {The oncology drug elesclomol selectively transports copper to the mitochondria to induce oxidative stress in cancer cells},
   journal = {Free Radical Biology and Medicine},
   volume = {52},
   number = {10},
   pages = {2142-2150},
   ISSN = {0891-5849},
   year = {2012},
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}

@article{RN186,
   author = {Chun, Haarin and Catterton, Tracy and Kim, Heejeong and Lee, Jaekwon and Kim, Byung-Eun},
   title = {Organ-specific regulation of ATP7A abundance is coordinated with systemic copper homeostasis},
   journal = {Scientific reports},
   volume = {7},
   number = {1},
   pages = {12001},
   ISSN = {2045-2322},
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}

@article{RN43,
   author = {Albayrak, Gulsah and Korkmaz, Funda Demirtas and Tozcu, Duygu and Dogan Turacli, Irem},
   title = {The outcomes of an impaired powerhouse in KRAS mutant lung adenocarcinoma cells by Elesclomol},
   journal = {Journal of cellular biochemistry},
   ISSN = {0730-2312},
   year = {2019},
   type = {Journal Article}
}

@article{RN231,
   author = {Anderson, Jennifer L and Morran, Levi T and Phillips, Patrick C},
   title = {Outcrossing and the maintenance of males within C. elegans populations},
   journal = {Journal of heredity},
   volume = {101},
   number = {suppl_1},
   pages = {S62-S74},
   ISSN = {1465-7333},
   year = {2010},
   type = {Journal Article}
}

@article{RN215,
   author = {Tümer, Zeynep},
   title = {An overview and update of ATP7A mutations leading to Menkes disease and occipital horn syndrome},
   journal = {Human mutation},
   volume = {34},
   number = {3},
   pages = {417-429},
   ISSN = {1059-7794},
   year = {2013},
   type = {Journal Article}
}
